current therapy for thoracic aortic diseasefactors, and/or a familial form of thoracic aortic...

Current Therapy for

Thoracic Aortic

Disease John R. Doty, M.D.

Intermountain Center for Aortic Disease

OBJECTIVES

• Review established guidelines for treatment of thoracic aortic disease

• Update on evaluation and diagnosis of aortic aneurysm and dissection

• Discussion of new and evolving techniques in surgical treatment of thoracic aortic disease

Thoracic Aortic Disease

• Thoracic aortic disease is usually asymptomatic until something acute and catastrophic occurs

• Imaging is the cornerstone of therapy • Expense, radiation, contrast

• Identification of higher risk individuals • Intervene before the catastrophe

Aortic Anatomy

• Root = aortic annulus and sinuses to the level of the sinotubular junction

• Ascending = sinotubular junction to innominate artery

• Arch = innominate artery to left subclavian • Descending = left subclavian to diaphragm

Aortic Anatomy

Root

Ascending Arch

Descending

The Aorta – Size Matters • The magic number is 4

Circulation 2010;121:1551

Genetics of Aortic Disease

• Marfan syndrome = fibrillin-1 • Loeys-Dietz syndrome = TGFBR • Ehlers-Danlos syndrome = COL3A1 • Familial aortic disease = vascular

smooth muscle cell contractile dysfunction

• Actin (ACTA2) • Myosin (MYH11)

Genetics of Aortic Disease

• Familial thoracic aortic disease • About 20% have affected relative • Most common gene alteration is ACTA2

(10-14% of families) • Autosomal dominant inheritance • Decreased penetrance in women • Presence of genetic mutation but lack of

syndrome features

Genetics of Aortic Disease

• Bicuspid aortic valve • The most common congenital heart

defect (0.9-1.4% of population) • Possible autosomal dominant pattern in

certain families • Loss of elastic fibers, cystic medial

necrosis, and altered smooth muscle alignment in aorta

• 9x increase in aortic dissection

Screening – Genetic Aortic Disease

• Obtain a careful family history if you suspect genetic aortic disease.

• Most patients do not need expensive serologic or tissue testing.

• CT and echocardiography are the mainstay of aortic imaging.

• Prompt referral to aortic center is indicated for any suspicious patient.

Screening – Familial Aortic Disease

• Class I: Aortic imaging is recommended for first-degree relatives of patients with aneurysm or dissection.

• Class IIa: If one or more first-degree relatives is found to have aneurysm or dissection, imaging of second-degree relatives is reasonable.

Screening – Bicuspid Aortic Valve

• Class I: First-degree relatives of patients with a bicuspid aortic valve, premature onset of thoracic aortic disease with minimal risk factors, and/or a familial form of thoracic aortic aneurysm and dissection should be evaluated for the presence of a bicuspid aortic valve and asymptomatic thoracic aortic disease.

• Class I: All patients with a bicuspid aortic valve should have both the aortic root and ascending thoracic aorta evaluated for evidence of aortic dilatation.

Indications for Ascending Aorta Replacement

• Class I indications • Symptomatic

• Size:

• Ascending 5.0-5.5 cm

• Marfan, bicuspid 4.0-5.0 cm

• Family history of dissection

• Rapid growth (>0.5cm/year)

• Concomitant AVR and ascending > 4.5 cm

Indications for Aortic Aneurysm Replacement

• AHA Guidelines for aortic pathology beyond the ascending aorta • Arch = 5.5 cm or greater • Descending chronic dissection = open

repair when 5.5 cm or greater • Descending degenerative or traumatic

= endovascular repair • Thoracoabdominal = 6.0 cm

Surveillance of Aortic Disease - AHA guidelines

• CT or MRI is appropriate • Repeat imaging for aortic dissection at

1 month, 3 months, 6 months, and annual

• Use same modality and same institution where possible

• MRI probably preferred in stable disease to reduce radiation over time

Medical Management

• Careful history: family history, bicuspid aortic valve, cerebral aneurysms, sudden/unexplained death

• Surveillance imaging • ß-blockers • ACE/ARB

• Losartan clinically decreases TGFß signalling

Ascending Aneurysm

Doty DB and Doty JR, Cardiac Surgery: Operative Technique, 2012

Ascending Aneurysm – Surgical Repair

Ascending Aorta - Current Outcomes

• Hess et al (Florida) • 44 patients underwent replacement

ascending aorta/hemiarch • Mean HCA = 12 minutes • No operative mortality, stroke, or renal

dysfunction

Aortic Root Aneurysm

Doty DB and Doty JR, Cardiac Surgery: Operative Technique, 2012

Aortic Root Aneurysm – Surgical Repair

Aortic Root and Ascending Aneurysm

Doty DB and Doty JR, Cardiac Surgery: Operative Technique, 2012

Root + Ascending Aorta – Surgical Repair

Root + Ascending Aorta – Current Outcomes

• Doty et al (Utah) • 50 patients underwent replacement of

root + ascending aorta/hemiarch • Mean HCA = 13 minutes • No operative mortality or stroke • 98% freedom from reoperation • No further aortic pathology

Marfan Aortic Root

Doty DB and Doty JR, Cardiac Surgery: Operative Technique, 2012

Valve-Sparing Root Replacement

Doty DB and Doty JR, Cardiac Surgery: Operative Technique, 2012

Valve-Sparing Root Replacement

Valve-Sparing Root - Current Outcomes

• Cameron et al (Johns Hopkins) • 19-year experience with valve-sparing

root replacement for Marfan syndrome • 127 patients • 1 death • No reoperations with David I • Very low thromboembolism and

endocarditis rates

Arch Aneurysm

Doty DB and Doty JR, Cardiac Surgery: Operative Technique, 2012

Arch Aneurysm – Reimplantation Repair

Aortic Arch - Current Outcomes

• Safi et al (Houston) • 1316 arch repairs over 20-year period • Operative mortality 10% • Stroke 3% • Higher risk in emergent, older, acute

dissection, and ruptured aorta

Descending Aneurysm

Doty DB and Doty JR, Cardiac Surgery: Operative Technique, 2012

Descending Aneurysm – Surgical Repair

Doty DB and Doty JR, Cardiac Surgery: Operative Technique, 2012

Descending Aneurysm – Surgical Repair

Descending Aneurysm - Current Outcomes

• Elefteriades et al (Yale) • 130 patients underwent open surgical

repair of descending thoracic aorta • Operative mortality 3% • Stroke 4%; paraplegia 7% • 5-year survival 73%

Thoracoabdominal Aneurysm

Thoracoabdominal Aneurysm Open Repair

Doty DB and Doty JR, Cardiac Surgery: Operative Technique, 2012

Thoracoabdominal Aneurysm Open Repair

Thoracoabdominal Aorta - Current Outcomes

• Kouchoukos et al (St. Louis) • 218 patients • Operative mortality 5% • Spinal cord injury 3.5% • Stroke 4% • Renal failure 7%

Thoracic Endovascular Aortic Repair (TEVAR)

• Potential benefits:

• Less invasive

• Avoidance of CPB/HCA

• Quicker recovery

• Reduction in stroke and paralysis

• Disadvantages:

• Failure to seal (endoleak)

• Inability to treat entire pathology in areas of branching or extensive aortic disease

Thoracic Endovascular Aortic Repair (TEVAR)

• Proven and preferred therapy for descending thoracic aneurysms and chronic dissections

• Recent FDA approval for treatment of acute type B aortic dissections

• Growing interest in hybrid and extended applications to complex aortic pathology

Doty DB and Doty JR, Cardiac Surgery: Operative Technique, 2012

Descending Aneurysm - Endovascular Repair

TEVAR – CT Scan

Descending Aneurysm - Endovascular

• Bavaria et al (Pennsylvania) • 140 patients underwent endovascular

stent-grafting of descending aneurysm • Operative mortality 2% • Spinal cord injury 3% • No difference in 2-year mortality

compared to open surgical cohort

Expanding Endovascular therapy

• New techniques and approaches with goals of decreasing mortality/complications, including long-term development of other aortic problems

• Combining open operations with endovascular strategies

• Example: “hybrid” arch repair • Sternotomy with debranching of head

vessels • Stent-graft into descending aorta

Type A Dissection – Arch Debranching

Hybrid arch debranching and endografting

• Roselli et al (Cleveland) • 17 hybrid arch repairs for acute type A

• Portion of stent-graft under arch cut out • Stent-graft manually sewn to head

vessels and to proximal open graft • No deaths • 2 strokes (6%) and 2 paralysis (6%)

Hybrid arch debranching and endografting

Aortic Dissection

Type A Type B

Aortic Dissection – CT

Type A Type B

Aortic Dissection – Type A

Aortic Dissection – Type B

Type A Dissection - Current Outcomes

• Pagni et al (Lousville) • 117 patients open surgical repair of

type A dissection 2003-2011 • Operative mortality 19% • 5-year survival 60%

Type B Dissection - Current Outcomes

• Estrera et al (Houston) • Medical management = 10% hospital

mortality • Open surgical repair = 17% hospital

mortality • 5-year survival 75%

Acute Aortic Dissection – Type B Endovascular

• DISSECTION trial data - STS 2014 • 20% presented with acute rupture • 100% technical success • 100% entry tear coverage • 8% all-cause mortality at 30 days • False lumen partial or complete

thrombosis in 91%

Expanding Endovascular Repair of Aortic Disease

• Aortic dissection • Chronic type B • Acute, complicated type B • Acute, uncomplicated type B

• Penetrating aortic ulcer • Intramural hematoma • Aortic transection

Aortic Dissection - Endovascular

• INSTEAD Trial for stable, uncomplicated type B dissection

• OMT vs. OMT + elective stent graft • No difference in 2-year mortality • Improved aortic remodeling after stent-

grafting (91% vs. 19%)

Intramural Hematoma - AHA Guidelines

• An intramural hematoma should be treated similar to aortic dissection in that portion of the aorta

• Treatment of penetrating aortic ulcer remains controversial, but endovascular repair is a reasonable approach

Spectrum of Dissection

Penetrating Ulcer - Endovascular

Penetrating Ulcer - Endovascular

Intermountain Center For Aortic Disease

• Development of collaborative program for treatment of all forms of aortic and great vessel disease

• Screening and ongoing follow-up of patients with known aortic pathology

• Shared expertise of cardiovascular surgery, radiology, vascular surgery, and cardiology for optimal care

Conclusions

• Once an aortic patient, always an aortic patient • High index of suspicion for screening • Early referral for elective care is the safest and

most effective treatment - 4.0 cm or larger, please refer early!

Intermountain Center for Aortic Disease 1-855-507-4223 801-507-4223

(507-ICAD)